title: Classification, natural history and staging of idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is defined as a fibrosing disease limited to the lungs of unknown aetiology characterised radiologically and/or morphologically by the usual interstitial pneumonitis pattern. It is a disease with an ominous prognosis. There is currently no consensus regarding the staging and classification of IPF. As highlighted in the revised ATS/ERS guidelines, proposed stages may be based on resting pulmonary function test measurements and/or the extent of radiologic abnormalities, but it is unknown if these staging approaches are relevant to clinical decision-making. Thus, the unmet needs in IPF include a complete knowledge of the pathogenetic mechanisms leading to lung destruction, an improved understanding of different clinico-radiological subtypes of this disorder and finally, the identification of staging systems of clinical value.
authors.affiliation: Department of Thoracic Diseases, Ospedale Morgagni Forlì, Italy. venerino.poletti@gmail.com
authors.forename: V
authors.initials: V
authors.lastname: Poletti
authors.forename: J
authors.initials: J
authors.lastname: Egan
mesh_terms: D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis; D008168:Lung; D011379:Prognosis; D011658:Pulmonary Fibrosis; D011871:Radiology; D012129:Respiratory Function Tests